Scimitar Syndrome Associated with Coarctation of Aorta

The scimitar syndrome is a rare congenital cardiopulmonary malformation. Its association with coarctation of aorta is extremely uncommon. Herein, we report a male infant with Scimitar syndrome associated with coarctation of aorta. He had symptoms of heart failure. Chest X-ray demonstrated hypoplastic right lung and dextroposition of the heart. 2-D echocardiography and color Doppler showed secundum atrial septal defect and coarctation of aorta. Cardiac catheterization revealed pulmonary hypertension and left to right shunt. Angiography revealed abnormal drainage of right pulmonary veins to inferior vena cava and the presence of eoarctation of aorta. Surgical correction of coarctation was attempted. In spite of repair of coarctation, the patient died due to respiratory insufficiency. We conclude that the degree of hypoplasia of the lung and severity of associated cardiovascular anomalies are İmportant in predicting prognosis of patients with the Scimitar syndrome.